Polycythemia vera pathophysiology
|
Polycythemia vera Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Polycythemia vera pathophysiology On the Web |
|
American Roentgen Ray Society Images of Polycythemia vera pathophysiology |
|
Risk calculators and risk factors for Polycythemia vera pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Please help WikiDoc by adding more content here. It's easy! Click here to learn about editing.
Overview
Pathophysiology
In primary polycythemia, there may be 8 to 9 million and occasionally 11 million erythrocytes per cubic millimeter of blood (a normal range for adults is 4-6), and the hematocrit may be as high as 70 to 80%. In addition, the total blood volume sometimes increases to as much as twice normal. The entire vascular system can become markedly engorged with blood, and circulation times for blood throughout the body can increase up to twice the normal value. The increased numbers of erythrocytes can cause the viscosity of the blood to increase as much as five times normal. Capillaries can become plugged by the very viscous blood, and the flow of blood through the vessels tends to be extremely sluggish.
Genetics
Recently, in 2005, a mutation in the JAK2 kinase (V617F) was found by multiple research groups [1][2] to be strongly associated with polycythemia vera. JAK2 is a member of the Janus kinase family. This mutation may be helpful in making a diagnosis or as a target for future therapy.
References
- ↑ Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, Vassiliou GS, Bench AJ, Boyd EM, Curtin N, Scott MA, Erber WN, Green AR (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365 (9464): 1054–61. PMID 15781101.
- ↑ Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, Boggon TJ, Wlodarska I, Clark JJ, Moore S, Adelsperger J, Koo S, Lee JC, Gabriel S, Mercher T, D'Andrea A, Frohling S, Dohner K, Marynen P, Vandenberghe P, Mesa RA, Tefferi A, Griffin JD, Eck MJ, Sellers WR, Meyerson M, Golub TR, Lee SJ, Gilliland DG (2005). "Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell. 7 (4): 387–97. PMID 15837627.